Classification and Description of Frontotemporal Dementias
Abstract
A bstract: A number of distinct clinical syndromes have been described that are associated with focal degeneration of the frontal and temporal lobes and have a non‐Alzheimer pathology. The nosological status of frontotemporal lobar degeneration (FTLD) has been a matter of controversy, in view of the diversity of clinical manifestations and distribution and nature of histopathological change. This paper describes the major clinical syndromes of frontotemporal dementia, progressive aphasia, and semantic dementia; it discusses their underlying pathologies and considers their molecular status. Common histopathological changes are demonstrated across the three clinical syndromes, highlighting the link between these clinical disorders. It has been suggested that these disorders should be regarded as tauopathies on the basis of the tau pathology seen in a number of cases and the mutations in the tau gene in some familial cases. However, in a series of 47 consecutive autopsy series of FTLD, only 36% had tau pathology and 10% mutations in the tau gene, suggesting that FTLD does not constitute a unitary etiological disorder and that its characterization as a tauopathy may be potentially misleading.
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The clinical distinction between fvAD and bvFTD has implications for prognosis, treatment, disease progression, and caregiver burden.
“The appropriate clinical distinction between fvAD and bvFTD has implications for prognosis, treatment, disease progression, and caregiver burden [11–15].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [11]
bvFTD presentation is characterized by changes in personality and behavior, most notably apathy, disinhibition, loss of self-awareness, and loss of empathy.
“Typically, the presentation is characterized by changes in personality and behavior, most notably apathy, disinhibition, loss of self-awareness, and loss of empathy [11, 18].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [11]
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“Examples include being overly familiar with strangers, making lewd comments, or having no empathy for others’ feelings, and impaired control of impulses may lead to hyperphagia, hypersexuality, or reckless spending.(14) It is understandable how such behaviors might initially be interpreted as a late-onset presentation…”
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“In semantic dementia, the link between words and their meanings is disrupted (Méligne et al., 2011; Patterson, 2014), making output fluent, but ‘empty of content’ (Neary, Snowden, & Mann, 2000, p. 48), and input difficult to understand (Hodges, Patterson, Oxbury, & Funnell, 1992, p. 1796).”
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“The subtypes of FTLD can be distinguished by the prominent symptoms, which relect the area afected by neuron loss [11, 12].”
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“It is responsible for at least 20% of presenile cases (Neary et al., 2000).”
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“Semantic dementia (SD) is a less common FTD subtype, which is characterized by language impairment, as well as declines in executive fucntioning (Neary et al., 2000; Seelaar et al., 2011).”
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“In effect, patients may experience changes in behavior, language presentation, and personality (Neary et al., 2000).”
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“The three major forms of FTD include the behavioral variant of FTD (bvFTD) and the two language forms of FTD, semantic dementia (SD) and progressive non-fluent aphasia (PNFA) ( Neary, Snowden, & Mann, 2000 ).”
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Estrogen Signaling Based Current and Potential Therapies Against Obesity and Related Diseases
Singh, S. K., & Singh, R. (2021) · Springer eBooks · doi:10.1007/978-981-16-1846-8_15 · Cited by 1 · see citing works
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- Authors
- NEARY, D., SNOWDEN, J. S., & MANN, D. M. A.
- Affiliations
- Manchester Royal Infirmary (GB)
- University of Manchester (GB)
- Corresponding author
- NEARY, D.
- Venue
- Annals of the New York Academy of Sciences
- Publisher
- Wiley
- Published
- 2000-12
- Volume
- 920
- Issue
- 1
- Pages
- 46-51
- ISSN
- 0077-8923 (ISSN-L)
- 1749-6632
- OpenAlex
- W2000757231
- Cited by
- 99
- References
- 12
- Citations by year
- 2025: 4 · 2024: 1 · 2022: 2 · 2021: 2 · 2020: 20 · 2019: 2 · 2018: 2 · 2017: 3 · 2016: 1 · 2015: 1 · 2014: 2 · 2012: 4
- FWCI
- 1.16
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of the New York Academy of Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- frontotemporal dementia, frontotemporal lobar degeneration, progressive aphasia, semantic dementia, tauopathy, tau gene mutations, disease classification, histopathology, molecular genetics, non-Alzheimer's dementia
- MeSH terms
- Aged, Aphasia, Dementia, Diagnosis, Differential, Female, Frontal Lobe, Humans, Male, Middle Aged, Mutation, Temporal Lobe, Motor Neuron Disease
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